Vitiligoid hypopigmented macules and tumor of the follicular infundibulum

Abstract

Tumor do infundíbulo folicular é lesão cutânea incomum, com várias formas de apresentação clínica. Destacam-se as formas solitária e eruptiva/múltipla. A primeira apresenta-se como lesão papulonodular descamativa em região de cabeça e pescoço. A segunda, alvo deste relato, é mais rara, ocorrendo em face, pescoço e porção superior do tronco como lesões maculares pardas, avermelhadas ou, mais frequentemente, hipopigmentadas, podendo levar ao diagnóstico diferencial com lesões vitiligóides. A apresentação microscópica das diferentes formas clínicas é similar. Neste artigo, relatamos um caso de tumor do infundíbulo folicular múltiplo/eruptivo apresentando-se como máculas vitiligóides faciais em homem de 35 anos.

Hipopigmentação; Neoplasias de anexos e de apêndices cutâneos; Neoplasias cutâneas

DERMATOPATHOLOGY

Vitiligoid hypopigmented macules and tumor of the follicular infundibulum

Alesso Cervantes Sartorelli^I; Francisco Eduardo M. Leite^II; Isabel Violeta de Carvalho Friedman^III; Horácio Friedman^IV

^IPathologist, Laboratório Diagnose - Brasilia (DF), Brazil

^IIDermatologist - Brasilia (DF), Brazil

^IIIPathologist, Laboratório Diagnose - Brasilia (DF), Brazil

^IVFull Professor, Department of Pathology, Medical School, Universidade de Brasilia - Brasilia (DF), Brazil

Mailing Address

ABSTRACT

Tumor of the follicular infundibulum is an uncommon cutaneous lesion with different forms of clinical presentation, namely solitary and multiple/eruptive variants. The former shows predilection for head and neck and presents as a papulonodular scaly tumor. The latter is less frequent and occurs on facial, neck and upper chest areas as brown, reddish or more commonly hypopigmented macules leading to the differential diagnosis with vitiligo. The different clinical forms share the same histopathologic aspect. The present report describes a case of the multiple variant of tumor of the follicular infundibulum disclosing facial vitiligoid macules in a 35-year-old male patient.

Keywords: Hypopigmentation; Neoplasms, Adnexal and skin appendage; Skin neoplasms

INTRODUÇÃO

Follicular infundibulum tumor (FIT) or infundibuloma, described in 1961 by Mehregan and Butler, is an uncommon tumor found in less than 10/100,000 skin biopsies, but it has different forms of clinical presentation: 1) solitary; 2) multiple or eruptive; 3) associated with Cowden’s disease, sebaceous nevus, Schopf-Schulze-Passarge syndrome, and 4) associated with other skin tumors ^1-7. Single lesion is common, preferably in middle-aged women and located on the head and neck, asymptomatic and papulonodular desquamative, measuring 1 to 2 cm simulating a basal cell carcinoma. The eruptive/ multiple form (infundibulomatosis), described by Kossard, is rare, with multiple lesions on the face, neck and upper portion of the trunk, symmetrical, with aspect of hypopigmented macules or papules, light brown or red ^{3, 8, 9}. Sun exposure accentuates the multiple hypopigmented lesions that remain like that while the surrounding skin is tanned. The solitary and multiple variants have similar microscopic aspect, with proliferation in fenestrated plaque of pale keratinocytes organized in anastomosed trabeculae connected to the epidermis. The present case illustrates the multiple hypopigmented lesions: the uncommon vitiligoid form of the tumor.

CASE REPORT

Male 35-year-old patient, had facial hypopigmented macules for 3 years, symmetric and asymptomatic. Wood’s glass highlighted the lesions (Figure 1), one of which was submitted to biopsy. Microscopic analysis revealed fenestrated plaque connected to the epidermis basis and formed by anastomosed trabeculae of pale keratinocytes (Figure 2A), containing glycogen inside them (Figure 2B). Fontana-Masson staining showed absence of melanin pigmentation in the lesion (Figure 3A). Adjacent epidermis showed preserved pigmentation (Figure 3B). CO2 laser management repigmentation and improvement of lesion texture. Later, we received skin biopsy result showing very similar microscopic aspect, which we learned was from the patient’s brother. The dermatologist learned that other family members had clinically similar lesions. However, the patient and his family members refused to undergo treatment or follow-up.

   

   

DISCUSSION

The present discussion is focused on the multiple form of FIT. Clinical aspect of normally different, enabling diagnostic suspicion ⁴. However, some cases have small and few lesions, difficult to visualize, which leads to underdiagnosing ⁴. FIT has microscopic aspect which is normally typical, with well circumscribed lesion, formed by pale keratinocytes, containing glycogen and proliferating from the epidermal basis so as to form a fenestrated plaque of anastomosed trabeculae. The desmoplastic variant and sebaceous or eccrine differentiation have been rarely described ^{2, 10, 11}. There are many elastic fibers on the adjacent dermis. Hair follicles present in the area are involved by the lesion but do not produce follicular structures. Both multiple and solitary cases are considered benign ³. Evident basal membrane, broad pale cytoplasm, and absence of mitosis, atypias and apoptosis normally facilitate the differential diagnosis with basal cell carcinoma. Immunohistochemical reaction to BerEp4 for basal cell carcinoma is negative for FIT .^{2, 6, 12, 13} However, the rare transformation of FIT into basal cell carcinoma has already been described ^{2, 3, 4, 14}. In the present case, the clinical presentation directed the differential diagnosis to macular hypopigmented lesions, including vitiligo. The histopathological aspect was typical of FIT - hypopigmentation could be explained by lack of melanin in the lesion, as shown by Fontana-Masson staining, previously described by Cribier and Grosshans.4 To bring together clinical suspicion of vitiligoid lesions with histopathological diagnosis of tumor lesion is a rare situation, but characteristics of FIT. Even though the genetic basis has not been established, the presence of more than one family member with histologically identical lesion and occasional association with other genetic-known syndromes favor the hypothesis of a possible underlying genetic defect ^{2, 3, 15}. It is recommendable to have periodical follow-up of patients, considering the impossibility of having complete treatment, because of the cosmetic implications and the remote possibility of transformation into basal cell carcinoma.

REFERENCES

• 1.  Mehregan AH, Butler JD. A tumor of follicular infundibulum. Report of a case. Arch Dermatol. 1961;83:924-7.
• 2.  Hurt MA, Kaddu S, Kutzner H, Cribier B, Schulz T, Hartschuh W. Benign tumours with follicular differentiation. In: LeBoit PE, Burg G, Weedon D, Sarasain A, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours. Lyon: IARC Press; 2006. p158.
• 3.  Cheng AC, Chang YL, Wu YY, Hu SL, Chuan MT. Multiple tumors of the follicular infundibulum. Dermatol Surg. 2004;30:1246-8.
• 4.  Cribier B, Grosshans E. Tumor of the follicular infundibu lum: a clinicopathologic study. J Am Acad Dermatol. 1995;33:979-84.
• 5.  Mehregan AH. Tumor of follicular infundibulum. Dermatologica. 1971;142:177-83.
• 6.  Patterson JW, Wick MR. Nonmelanocytic tumors of the skin. Washington, DC: ARP Press; 2006. p.71-2.
• 7.  Inaloz HS, Inaloz SS, Unal B, Eralp A. Coexistence of tumor of the follicular infundibulum with an unusual trichilemmal tumor. Am J Dermatopathol. 2002;24:406-8.
• 8.  Kossard S, Kocsard E, Poyzer KG. Infundibulomatosis. Arch Dermatol. 1983;119:267-8.
• 9.  Stefanaki C, Stefanaki K, Kontochristopoulos G, Nikitidou S, Tzermias C, Katsambas A. A case of eruptive infundibu lomas. Dermatology. 2003;207:424-5.
• 10.  Mahalingam M, Bhawan J, Finn R, Stefanato CM. Tumor of the follicular infundibulum with sebaceous differentiation. J Cutan Pathol. 2001;28:314-7.
• 11.  Horn TD, Vennos EM, Bernstein BD, Cooper PH. Multiple tumors of follicular infundibulum with sweat duct differentiation. J Cutan Pathol. 1995;22:281-7.
• 12.  Klein W, Chan E, Seykora JT. Tumors of the epidermal appendages. In: Elder DE, ed. LeverÂ's histopathology of the skin. Philadelphia: Lippincott Williams & Wilkins ; 2005. p. 885-6.
• 13.  Weedon D. Skin pathology. London: Churchill Livingstone; 1997. p.718-9.
• 14.  Schnitzler L, Civatte J, Robin F, Demay C. Multiple tumors of the follicular infundibulum with basocellular degenera tion. Apropos of a case. Ann Dermatol Venereol. 1987;114:551-6.
• 15.  Kolenik SA 3rd, Bolognia JL, Castiglione FM Jr, Longley BJ. Multiple tumors of the follicular infundibulum. Int J Dermatol. 1996;35:282-4.

*

Trabalho realizado no Laboratório Diagnose - Brasília (DF), Brasil.

Publication Dates

• Publication in this collection
  23 June 2009
• Date of issue
  Feb 2009

History

• Accepted
  30 Jan 2009
• Received
  30 Jan 2009